DSA 4: Jaundice

Question 1

when do you see clinical jaundice? what happens if there is hemolysis alone?

Answer 1

clinical jaundice: bilirubin >= 3 mg/dL (M>F, white & hispanic> african american)

hemolysis alone - bilirubin > 7 mg/dL

Question 2

What is the DDx for unconjugated jaundice

Answer 2

hemolytic syndrome - anemia/reaction

gilbert syndrome

crigler-najjar syndrome

viral hepatitis (seen in both)

Question 3

What is the DDx for conjugated jaundice

Answer 3

hepatitis - acute/chronic; infxous/non-infxous

cirrhosis

obstruction - choledochilothiasis, cholangitis, PBC, PSC, budd-chiari, pancreatic CA

dubin-johnson syndrome

rotor syndrome

drug reaction

pregnancy

Question 4

what are initial steps in evaluating the pt w/ jaundice ?

Answer 4

hyperbilirubinemia - conjugated or unconjugated

if other biochem liver test is abnormal

Question 5

what are diagnostics of conjugated vs unconjugated jaundice

Answer 5

chem labs - AST/ALT, total bilirubin, ALP

fractionated bilirubin = indirect or direct

fractionate ALP by ordering GGT - if GGT elevated = MCC liver source; if normal then consider bone or other source

conjugated: viral hep serology, AMA, ceruloplasmin, Fe studies, lipase/amylase; typically begin w/ US

unconjugated: CBC - (hemolysis=haptoglobin, reticulocyte cout, LDH); leukocytosis; peripheral smear

Question 6

what are possible causes of jaundice?

(draw a tree chart)

Answer 6

Question 7

What labs are seen in unconjugated hyperbilrubinemia (prehepatic)

Answer 7

CBC - anemia & thrombocytopenia

if bc of hemolysis - haptoglobin, reticulocyte count and LDH

peripheral smear - schistocytes? sickled cells?

Question 8

what are characteristic of gilbert syndrome

Answer 8

benign, asymptomatic

hereditary: reduced activity of uridine diphoshate glucuronyl transferase

fasting –> indirect (unconjugated) hyperbilirubinemia

No Tx required

reduced mortality from CV dz

Question 9

what are characterisitics of Dubin-Johnson syndrome?

clinical?

imaging?

grossly?

Bx?

Prognosis?

Answer 9

reduced excretory fxn of hepatocytes (mutation in ABCC2 gene)

=conjugated (direct) hyperbilirubinemia

benign, asymp hereditary jaundice

GB not visualized on oral cholesystography

liver = dark pigmented

Bx = centrilobular brown pigment

good prognosis

Question 10

what are characteristics of Rotor syndrome

Answer 10

reduced hepatic reuptake of bilirubin conjugates (mutation of OATP1B1 and OATP1B3)

conjugated (direct) hyperbilirubinemia

similar to dubin-johnson but liver is not pigmented & GB is visualized on oral cholecystography

prognosis - excellent

Question 11

what are characterisitics of intrahepatic cholestasis of pregnancy

Answer 11

cholestasis

= predom conjugated (direct) hyperbilrubinemia

benign cholestatic jaundice

3rd trimester

itching, GI symptoms, abn liver excretory fxn tests

Bx: cholestasis

prognosis = excellent

can have recurrance w/ subsequent pregnancies and use of OC